Primary pleural leiomyosarcoma with rapid progression and fatal outcome: a case report
© Rais et al; licensee BioMed Central Ltd. 2012
Received: 6 December 2011
Accepted: 5 April 2012
Published: 5 April 2012
Leiomyosarcomas are neoplasms of smooth muscles that most commonly arise from the uterus, gastrointestinal tract, or soft tissue. Primary pleural leiomyosarcoma is extremely rare. To the best of our knowledge, only nine cases have been published to date. Because of the rarity of pleural leiomyosarcoma and its similarity (clinical and histological) to other pleural neoplasms, particularly sarcomatous mesothelioma, diagnosis is often difficult.
A 58-year-old North African man was admitted with complaints of dyspnea and chest pain to our hospital. Chest computed tomography revealed right pleural effusion and pleural thickening. A transthoracic needle biopsy yielded a diagnosis of leiomyosarcoma, and tumor cells were strongly and uniformly positive for vimentin, a smooth muscle actin at immunohistochemical analysis. A general examination did not show any metastatic lesions in other areas. One month after diagnosis, the tumor grew rapidly, with pulmonary invasion, and therefore he was treated only by palliative care. He died from respiratory failure one month later. Because no organ of origin of the leiomyosarcoma, other than the pleura, was detected, this case was diagnosed as a primary pleural leiomyosarcoma.
Although leiomyosarcoma originating from the pleura is rare, this entity is increasingly described. The purpose of presenting this case report is to raise awareness among clinicians to consider this clinical entity as a differential diagnosis when a pleural mass is identified.
Leiomyosarcomas are cancers of smooth muscle cells that can arise from any location but occur most often in the uterus, retroperitoneum, or intra-abdominal region. Primary pleural leiomyosarcomas are extremely rare [1, 2]. There are few reports of individual cases [1–5], but no large series from a single institution has been published. On radiological examination, primary pleural leiomyosarcoma presents as a mass or a pleural effusion. Metastasis is uncommon and typically occurs late in the disease process, underscoring the importance of early detection. When primary pleural leiomyosarcoma is seen in the pleura, it is important to establish whether the tumor originated in the lung or is secondary to metastases from a separate location, the latter of which is much more common.
To definitively establish the pleura as the primary site for leiomyosarcoma, all other possible sites of origin within the body must be excluded. Therefore, diagnosis is difficult and management is complete resection of the tumor if feasible [4, 6, 7]. In this report, a rare primary pleural leiomyosarcoma exhibiting rapid growth and fatal outcome is described. The diagnostic approach to our patient is presented and the pertinent literature is reviewed.
Leiomyosarcomas are neoplasms of smooth muscles that most commonly arise from the uterus, gastrointestinal tract, or soft tissue. Pleural leiomyosarcomas originating in the serosal membranes are even more unusual and represent controversial entities [1–3]. Sometimes, these tumors are classified as sarcomatous types of mesothelioma when they are diffuse and as variants of solitary fibrous tumors when they are localized [3, 5].
To the best of our knowledge, no large series has documented the exact number of pleural leiomyosarcoma cases worldwide. Data are available in isolated case reports and small case series. To the best of our knowledge, fewer than 10 cases have been reported [4–6]. Thanks to increasing awareness of this entity and increasing application of adjunctive diagnostic tools, more cases will be diagnosed in the future.
Clinically, pleural leiomyosarcoma resembles other primary tumors of the pleura in symptomatology (dyspnea, chest pain, or cough), physical signs, and radiology [5, 6]. On radiological examination, a mass lesion or pleural effusion or both are common, as in our case. In some cases, the tumor encases the lung, similarly to mesothelioma [5, 7].
Extrathoracic primary tumors should be excluded by physical examination and CT. Most pleural leiomyosarcomas are metastatic from other sites, including the gastrointestinal tract, uterus, retroperitoneum, and lung [1–4]. So exclusion of metastatic leiomyosarcoma in the pleura is an essential step in diagnosing the primary lesion. In all reported cases, a localized unilateral pleural lesion with no abnormality detected on careful examination of the soft tissue, gastrointestinal tract, and genitourinary tract suggested primary disease of the pleura [2–7]. The lesion in this case report is primary pleural leiomyosarcoma because imaging modalities, including CT, revealed no tumors other than the pleural tumor.
On reviewing the literature on pleural leiomyosarcoma, we found that most of the cases were diagnosed by surgical biopsy at either thoracoscopy or thoracotomy [1, 3]. Occasionally, the diagnosis has been made by CT-guided transthoracic biopsy, according to isolated reports [4, 7]. Histological examination supplemented with IHC staining studies is the most reliable and conclusive method of diagnosing leiomyosarcoma and differentiating it from other more frequent primary pleural malignancies, such as malignant mesothelioma (sarcomatous), malignant fibrous histiocytoma, solitary fibrous tumor of pleura, synovial sarcoma, and neurogenic tumors.
Microscopically, leiomyosarcomas are characterized by malignant spindle cells with cigar-shaped nuclei and scant fibrillary cytoplasm. Variable mitotic activity is observed [1, 4, 5]. However, because of the rarity of pleural leiomyosarcoma and its similarity (clinical and histological) to other pleural neoplasms, particularly sarcomatous mesothelioma, pathological diagnosis is often difficult.
Therefore, the use of an antibody panel is recommended in order to confirm the diagnosis of pleural leiomyosarcoma and to rule out a commonly considered differential diagnosis. Immunohistochemically, leiomyosarcomas are nearly uniformly positive for smooth muscle actin, desmin, and vimentin and negative for calretinin, carcinoembryonic antigen, cytokeratin, leukocyte common antigen, neuroendocrine filament, CD-117, and S-100 protein [4, 5]. In our case report, histological and immunohistochemical characteristics of the tumors studied were basically similar and conformed to those described by other authors.
Histopathology and IHC staining have been supplemented by cytogenetic analyses, which can confirm the diagnosis of leiomyosarcoma . Cytogenetic studies of the smooth muscle tumor of the pleura have revealed contained chromosomal losses at 10q and 13q, which are the most common losses described in soft tissue leiomyosarcomas .
The optimal treatment for pleural leiomyosarcoma has not been defined. Surgery is the mainstay of therapy for pleural sarcoma in limited disease, and even in some cases having local spread, resection was feasible [1, 6, 7, 9]. Multi-modal therapy, including surgery and radiation, has also been used. The need for adjuvant radiation or chemotherapy may be assessed on a case-by-case basis depending on the histological grade of the tumor and the clinical stage at the time of diagnosis . In the literature, the most common treatment is surgical resection alone or surgery with adjuvant radiation therapy [4–7].
As with leiomyosarcomas of other soft tissue sites, histological grade and clinical stage of the disease are the best prognostic factors. Patients with unresectable sarcoma or metastatic disease are treated with chemotherapy . Leiomyosarcomas are chemosensitive to doxorubicin, ifosfamide, and trabectedin, and the overall response rate is approximately 20% . The median survival for patients with extensive disease is about 12 months [10, 11]. In our case report, pleural leiomyosarcoma was misdiagnosed as pleural tuberculosis because of the frequency of this infection in Morocco. The diagnosis of pleural leiomyosarcoma was made later, after deterioration of the general condition of our patient and aggressive progression of the tumor, which was unresectable.
The addition of our case to the literature offers new clinicopathological data useful for better defining the diagnosis and biological behavior of pleural leiomyosarcoma. The purpose of presenting this case report is not only to report an uncommon pleural tumor but also to raise awareness among clinicians adding this clinical entity as a differential diagnosis when a pleural mass is identified.
Written informed consent was obtained from the patient's next of kin for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
We would like to thank the department of pathology at the Hôpital Universitaire International Cheikh Zaid for their cooperation and help in making and printing the slides.
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